Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive neurometabolic synthesis disease with sympathetic autoregulation deficiency. Perioperative management of AADCD presents unique challenges due to autonomic dysfunction, impaired sympathetic regulation of heart rate, and potential airway complications. Herein, we report the successful anesthetic management of a 14-year-old male patient with AADCD under total intravenous anesthesia during extensive dental procedures. We applied the rapid sequence induction and intubation method with cricoid pressure to minimize the risk of pulmonary aspiration due to the potential difficulty in mask ventilation and intubation, limited mouth opening, microcephaly, micrognathia, and poor cooperation. Intubation was performed without any complications. Due to the autonomic dysfunction, potential airway complications, and developmental, mental, and motor delays in this disorder, perioperative management requires a multidisciplinary approach with comprehensive preoperative evaluation.

Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive neurometabolic synthesis disease with sympathetic autoregulation deficiency. Perioperative management of AADCD presents unique challenges due to autonomic dysfunction, impaired sympathetic regulation of heart rate, and potential airway complications. Herein, we report the successful anesthetic management of a 14-year-old male patient with AADCD under total intravenous anesthesia during extensive dental procedures. We applied the rapid sequence induction and intubation method with cricoid pressure to minimize the risk of pulmonary aspiration due to the potential difficulty in mask ventilation and intubation, limited mouth opening, microcephaly, micrognathia, and poor cooperation. Intubation was performed without any complications. Due to the autonomic dysfunction, potential airway complications, and developmental, mental, and motor delays in this disorder, perioperative management requires a multidisciplinary approach with comprehensive preoperative evaluation.

Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive neurometabolic synthesis disease with sympathetic autoregulation deficiency. Perioperative management of AADCD presents unique challenges due to autonomic dysfunction, impaired sympathetic regulation of heart rate, and potential airway complications. Herein, we report the successful anesthetic management of a 14-year-old male patient with AADCD under total intravenous anesthesia during extensive dental procedures. We applied the rapid sequence induction and intubation method with cricoid pressure to minimize the risk of pulmonary aspiration due to the potential difficulty in mask ventilation and intubation, limited mouth opening, microcephaly, micrognathia, and poor cooperation. Intubation was performed without any complications. Due to the autonomic dysfunction, potential airway complications, and developmental, mental, and motor delays in this disorder, perioperative management requires a multidisciplinary approach with comprehensive preoperative evaluation.